[Primary cardiac lymphoma: diagnosis and treatment. Report of 6 cases and review of the literature]

Abstract

Primary cardiac lymphoma (PCL) is the rarest primary cardiac tumour and carries a poor prognosis. Early diagnosis, often difficult, to introduce appropriate treatment as soon as possible, seems to have a positive impact on prognosis. The authors report their experience of 6 patients with PCL. None of the patients had immune depression. The presentations were tamponade (N= 2), right heart failure (N= 1), general ill health (N= 3). A PCL was suspected on echocardiography and thoracic CT scan showing tumour invading the right heart chambers in all cases. The diagnosis of PCL was confirmed by surgical biopsy in 5 patients and by endomyocardial biopsy in 1 patient. A diffuse large cell type B lymphoma was found in 5 patients and an anaplastic lymphoma in 1 patient. One patient died of right heart failure 4 days after diagnosis and before starting chemotherapy. All the other patients received chemotherapy. Two patients died during their first course. The other three patients had several courses of chemotherapy: there are two survivors 17, 5 months later and one patient died 62 months after diagnosis. The diagnosis of PCL should be suspected in patients with a cardiac tumour associated or not with pericardial effusion. Early, appropriate chemotherapy seems to have a positive impact on the prognosis, justifying aggressive approaches to obtain a rapid histological diagnosis.